Lymphedema

Background

• Lymphedema is defined as the impaired ability of the lymphatic vasculature (congenital or acquired) to collect and transport interstitial fluid, resulting in the characteristic swelling of tissues (most commonly in the extremities).
• Primary lymphedema results from an inherent defect in lymph-carrying conduits due to a broad range of genetic syndromes and disorders.
• Secondary lymphedema is a result of an acquired defect or blockage in lymph-carrying conduits due to a broad range of extrinsic (acquired) disorders.
• The incidence and prevalence of lymphedema is dependent upon the type and geographical location:
  • Primary lymphedema has a reported prevalence of 1 in 6,000 to 10,000 live births.
  • Secondary lymphedema due to lymphatic filariasis (the most common worldwide cause in certain developing geographic regions) has a reported prevalence of greater than 120 million people worldwide. Incidence and prevalence of secondary lymphedema due to other causes varies based on specific secondary cause.

General Evaluation

• The diagnosis is usually based on a detailed clinical history and physical examination.
• Patient history may reveal causes of lymphedema, such as family history of genetic syndromes and disorders associated with primary lymphedema, circumferential wounds to extremity, recurrent bacterial infections, and malignancy or malignancy-related treatments such as surgical lymph node removal.
• The physical exam of skin and extremities demonstrates edema (typically pitting in early stages) which, in later stages, may be nonpitting and associated with fibrotic changes with adipose tissue accumulation.
• Perform radionuclide lymphoscintigraphy if diagnosis remains unclear.

Evaluation by Type

• For patients with lymphedema due to suspected filariasis:
  • Consider Infectious Disease consultation or someone experienced in managing filariasis.
  • Testing options include (options for testing may vary by institutional availability):
    • Blood smear to evaluate for presence of microfilariae (specimen ideally collected at night [10 PM-2 AM] due to nocturnal periodicity in some species).
    • Serologic enzyme immunoassays, which may be performed without regard to the time of day the specimen is collected.
    • Tissue specimen sample for visualization of adult worms or microfilariae.

Management

• Offer complex decongestive physiotherapy (CDPT) to patients with lymphedema. CDPT consists of 2 phases:
  • Phase 1 - manual lymphatic drainage, skin care, range-of-motion exercises, and compression with multi-layered bandage wrapping
  • Phase 2 - use of fitted garments to prevent re-accumulation of fluid after CDPT, continued skin care, and remedial exercise and repeated light massage as needed
• Medications (including diuretics) are generally not considered useful in the treatment of lymphedema; diuretics are only indicated if there is a specific comorbidity present which requires diuretic treatment.
• Consider surgery only for patients who are unresponsive to other therapies such as CDPT and have a compelling indication such as significant impairment of quality of life or suffering from psychosocial distress due to appearance of affected area.
• Other management may include exercise and weight reduction (particularly if obesity is a contributing factor).